Abstract

GOMEZ, ANA MARÍA et al. Langerhans cell histiocytosis in children. A description of 10 cases. CES Med. [online]. 2013, vol.27, n.2, pp.177-184. ISSN 0120-8705.

Introduction: Langerhans cell histiocytosis is a rare disease with different clinical and pathological presentation, is clinically divided into three groups: unifocal, multifocal unisystem, and multifocal multisystem Langerhans cell histiocytosis is a consequence of proliferation, accumulation and infiltration of cells that are part of the mononuclear phagocyte system in different organs. The goal with the description of this cases series is to highlight the importance and awareness to a timely diagnosis and management of this entity, for a better quality of life and longer survival. Methods: Retrospective study in pediatric patients diagnosed in the Valle del Lili Foundation over a period of six years. Results: In our series of cases there was no gender difference and the mean age at presentation was 33 months. In the diagnosis by immunohistochemistry identified Langerhans Cell Histiocytosis, the cases were classified as multisystemic -multifocal in 5 patients, unisystemic in 1 patients - unifocal unisystemic in 1 patients -multifocal eosinophilic granuloma in 3 patients. Of the 10 patients 5 had risk organ involvement and 1 died in the observation period. Discussion: la histiocitosis de células de Langerhans es una enfermedad de compromiso sistémico muy poco frecuente en pediatría, de diversas formas de presentación clínica, lo que la hace una entidad de difícil diagnóstico y manejo. La inmunohistoquimica es la mejor forma de diagnóstico en esta entidad

Keywords : Histiocytosis; Langerhans cells; Pediatrics.

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